Elsevier

The Lancet

Volume 372, Issue 9634, 19–25 July 2008, Pages 234-245
The Lancet

Seminar
Polymyalgia rheumatica and giant-cell arteritis

https://doi.org/10.1016/S0140-6736(08)61077-6Get rights and content

Summary

Polymyalgia rheumatica and giant-cell arteritis are closely related disorders that affect people of middle age and older. They frequently occur together. Both are syndromes of unknown cause, but genetic and environmental factors might have a role in their pathogenesis. The symptoms of polymyalgia rheumatica seem to be related to synovitis of proximal joints and extra-articular synovial structures. Giant-cell arteritis primarily affects the aorta and its extracranial branches. The clinical findings in giant-cell arteritis are broad, but commonly include visual loss, headache, scalp tenderness, jaw claudication, cerebrovascular accidents, aortic arch syndrome, thoracic aorta aneurysm, and dissection. Glucocorticosteroids are the cornerstone of treatment of both polymyalgia rheumatica and giant-cell arteritis. Some patients have a chronic course and might need glucocorticosteroids for several years. Adverse events of glucocorticosteroids affect more than 50% of patients. Trials of steroid-sparing drugs have yielded conflicting results. A greater understanding of the molecular mechanisms involved in the pathogenesis should provide new targets for therapy.

Section snippets

Definitions and diagnostic criteria

Giant-cell arteritis mainly involves the large-sized and medium-sized arteries, especially branches of the proximal aorta. Polymyalgia rheumatica is characterised by aching and morning stiffness in the shoulder and pelvic girdles and neck. The two disorders can occur separately or together in people aged 50 years and older.

The diagnosis of polymyalgia rheumatica depends on a combination of clinical symptoms, raised acute-phase reactants, exclusion of other diseases, and response to

Epidemiology

The incidence rates of giant-cell arteritis and polymyalgia rheumatica increase progressively after 50 years of age.13 The reported rates for giant-cell arteritis are highest in northern European countries and in Minnesota (USA), which has a population of similar ethnic background, and are 20 or more per 100 000 people older than 50 years.14, 15, 16 Rates of this disease are lower in Mediterranean countries17, 18 and lowest in Arabian and Asian countries.19, 20 The lowest prevalence was

Relation between giant-cell arteritis and polymyalgia rheumatica

The clinical connections between polymyalgia rheumatica and giant-cell arteritis have suggested that they are different manifestations of the same disease process. These connections include their frequent occurrence together, the older age at onset with progressively increasing incidence rates after 50 years, similar sex ratio, substantial increase of acute-phase reactants before treatment, and rapid responsiveness to glucocorticosteroids and outcome.13 Population-based studies have shown that

Pathology and pathogenesis

In giant-cell arteritis, inflammation mainly affects the large-sized and medium-sized muscular arteries, especially the proximal aorta and its branches.13, 31 These arteries have a prominent internal elastic membrane and vasa vasorum. As the cervical arteries penetrate the dura they become thinner, have much less elastic tissue, and no vasa vasorum. Intracranial arteries are rarely associated with the vasculitic process.32

The classic histological picture of giant-cell arteritis is characterised

Polymyalgia rheumatica

Polymyalgia rheumatica is typically characterised by aching and stiffness in the morning in the neck, shoulder, and pelvic girdles. Typically the stiffness in the morning lasts 30 min or more. Shoulder pain is the presenting finding in 70–95% of patients, whereas hips and neck are less frequently involved (50–70%). The pain usually radiates distally towards the elbows and knees. It can begin in one shoulder or hip, but soon becomes bilateral. Occasionally the symptoms begin more peripherally.

Laboratory findings and imaging

Laboratory findings in both polymyalgia rheumatica and giant-cell arteritis are non-specific but indicate the inflammatory nature of these syndromes. An ESR of at least 40 mm/h has been included in all sets of criteria for the diagnosis of polymyalgia rheumatica.1, 2, 3 However, a normal ESR has been reported in 7–20% of the patients with polymyalgia rheumatica.79 The American College of Rheumatology classification criteria for giant-cell arteritis include an ESR of 50 mm/h or more.4 However,

Differential diagnosis

Several disorders can mimic polymyalgia rheumatica.97 Peripheral arthritis, particularly affecting both hands, can pose a challenge in the distinction of polymyalgia rheumatica from elderly-onset rheumatoid arthritis. Pronounced symmetrical peripheral synovitis, positive rheumatoid factor and anticyclic citrullinated peptide antibodies, and the development of joint erosions and extra-articular manifestations differentiate rheumatoid arthritis from polymyalgia rheumatica. Follow-up is sometimes

Giant-cell arteritis

Glucocorticosteroids are the treatment of choice. Adequate doses quickly suppress clinical manifestations of this disorder and prevent most further ischaemic complications. If visual loss has occurred before start of treatment, it is not usually reversed.13, 105, 106 Glucocorticosteroid therapy should be initiated as soon as the diagnosis of giant-cell arteritis is established. We recommend an initial dose of 40–60 mg per day of prednisone (or equivalent) as a single or divided dose.13 If the

Future perspectives

The development of standardised classification and diagnostic criteria would help in a comparison of studies from different centres and assist clinicians. Additional investigation is needed about the use of pulse glucocorticosteroids at the onset of treatment for giant-cell arteritis to confirm whether this regimen reduces toxic effects of glucocorticosteroids. Identification of risk factors for extended and relapsing disease might allow a more effective use of glucocorticosteroids and could

Search strategy and selection criteria

We searched the Cochrane Library, Medline, and EMBASE, mainly with the search terms “polymyalgia rheumatica [Mesh]”, “giant cell arteritis [Mesh]”, and “temporal arteritis [Mesh]”. We largely selected articles published in English during the past 5 years, without excluding older papers that we considered to be highly relevant to the topics discussed in this Seminar. We also searched the reference lists of articles identified by this search strategy, and selected those that we judged

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